In this review, we summarize the clinical presentations of the acute stage of anti-N-methyl-
d
-aspartate (NMDA) receptor encephalitis and the neurocritical care strategy in intensive care units. Anti-NMDA receptor encephalitis has characteristic clinical features and is predominantly seen in young adults and children. Most patients have five stages of clinical presentation, including a prodromal phase, psychotic and/or seizure phase, unresponsive and/or catatonic phase, hyperkinetic phase, and gradual recovery phase. The clinical course usually begins with viral infection-like symptoms that last for up to 2 weeks (prodromal phase), followed by the rapid development of schizophrenia-like psychiatric symptoms and seizures (psychotic and seizure phase). Patients may have a decreased level of consciousness with central hypoventilation, frequently requiring mechanical ventilation. In the subsequent hyperkinetic phase, patients present with orofacial-limb dyskinesia and autonomic instability. Children with significant neurological symptoms of anti-NMDA receptor encephalitis should initially be managed in a pediatric intensive care unit. The acute critical presentations are, refractory seizures, autonomic dysfunction, hypoventilation, cardiac arrhythmia, and hyperkinetic crisis. Symptom-guided therapies and critical care are necessary in the acute stage to improve the prognosis.